Long term outcome in non-multiple sclerosis paediatric acquired demyelinating syndromes

Abstract

ObjectivesWe aimed to study the risks of relapse and long term disability in children with non-MS acquired demyelinating syndromes (ADS). MethodsIn this prospective, multi-centre study, from the 14 UK pediatric neurology centres, children (<16 years) experiencing a first episode of ADS were recruited from 2010-2014. Case report forms were collected prospectively. ResultsA total of 269 children were recruited and followed up for a median of 7.2 years. Median age at onset was 9y (IQR 9.5-14.5, 126 females). At last follow-up, 46 (18%) had MS, 4 AQP4-Ab NMOSD and 206 (80%) had other ADS, of which 27 (13%) relapsed. Relapsing MOGAD was the diagnosis in 12/27, 6 were seronegative and 9 did not have antibodies tested. Frequency of relapse differed according to first presentation in non-MS ADS, being least likely in transverse myelitis (p=0.025). In the non-MS group, MOG-Ab was predictive of relapse (HR=8.42; p<0.001) occurring 8 times as often in the first year and decreasing over time. Long-term difficulties did not differ between children with monophasic vs relapsing diseases. ConclusionThe risk of relapse in non-MS ADS depends on initial diagnosis, and MOG-Ab positivity. Long-term difficulties are observed regardless of relapses and are determined by presenting phenotype.